Endothelial and Epithelial-Like Cell Formations in a Case of Posterior Polymorphous Dystrophy

Richardson, Walter P.; Hettinger, Michael E.

doi:10.1001/archopht.1985.01050100096028

Cited by 15 publications

(1 citation statement)

References 11 publications

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“…Moreover, changes in the posterior stroma and basal membrane of the epithelium were also described in some patients ). Proliferating epithelial-like endothelial cells with prominent microvilli, abundant keratofibrils, desmosomes and sparse microorganelles (all characteristics of epithelial cells) are the most common findings in PPCD corneas (Boruchoff and Kuwabara 1971;Feil et al 1997;Krachmer 1985;Matsumoto et al 1988; Richardson and Hettinger 1985;Rodrigues et al 1981). Epithelization was also confirmed by the detection of cytokeratins (CKs), typical epithelial proteins Rodrigues et al 1980).…”

Section: Introductionsupporting

confidence: 45%

Recurrence of posterior polymorphous corneal dystrophy is caused by the overgrowth of the original diseased host endothelium

Merjava

Malinova

Lišková

et al. 2011

Histochem Cell Biol

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Posterior polymorphous corneal dystrophy (PPCD) is a rare, bilateral autosomal dominant disorder affecting primarily the corneal endothelium and descemet membrane (DM). The aim of this study was to establish the origin of abnormal endothelium in a patient with PPCD exhibiting cornea graft failure after keratoplasty surgery. A sex-mismatched graft obtained from a patient with PPCD who underwent repeat penetrating keratoplasty and the patient's original cornea were investigated. Combined fluorescent immunohistochemistry for cytokeratin (CK) 19 (a marker of aberrant PPCD endothelium) with fluorescence in situ hybridization (FISH) of the sex chromosomes were used in order to characterize the cells on the posterior graft surface. The pathological endothelium of the failed PPCD cornea revealed strong positivity for CK19 using fluorescent immunohistochemistry. In all the CK19-positive cells, both X and Y chromosomes were simultaneously detected using FISH. The results clearly showed the original cells of the patient (XY), within 3.5 years, almost totally overgrown the posterior corneal surface of the graft (XX). Moreover, an abnormal posterior collagenous layer populated by fibroblast-like cells was observed between DM and the endothelium in the failed graft, but its exact origin could not be established due to the low number of cells. Simultaneous detection of CK19 using fluorescent immunohistochemistry together with the detection of gonosomes using FISH was performed for the first time in the cornea and allowed us to prove that the recurrence of PPCD was caused by pathological abnormal proliferation and migration of recipient cells into donor graft.

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